Department of Dermatology, Silesian Medical University, Francuska Street 20/24, Katowice, Poland.
Diagn Pathol. 2009 Aug 28;4:28. doi: 10.1186/1746-1596-4-28.
Necrobiosis lipoidica diabeticorum is a rare disease of unclear etiology, that occurs in about 1% of diabetic patients.
We present case of granulomatosis disciformis chronica et progressiva Miescher with good response to systemic corticosteroids therapy.Patient 45 years old woman, with primary yellow-brown areas skin lesions, with foci well separated from surroundings on both lower legs, that occurred 5 years ago. In laboratory tests there was no abnormalities. Because of advance suggestion (after last admit in dermatological ward) of observation according to xantogranuloma necrobioticum tests for paraproteinemia were made. Immunoelectrophoresis, IgG, IgM, IgA levels, kappa light chain, lambda heavy chain; were correct, Bence-Johns protein-negative. During hospitalization in Clinic methylprednisolone in dose of 32 mg od, vascular drugs and local steroidotherapy was applied with good therapeutic response.
We described case of typical clinical and histological characters of necrobiosis lipoidica. without diabetes-granulomatosis disciformis chronica et progressiva Miescher that despite of suspicion of proper diagnosis for a long time was not treat effective.
糖尿病性脂性渐进性坏死是一种病因不明的罕见疾病,约发生在 1%的糖尿病患者中。
我们报告了一例米舍尔慢性进行性盘状肉芽肿性脂膜炎,全身皮质类固醇治疗反应良好。患者为 45 岁女性,下肢有原发性黄棕色皮肤病变,病灶与周围界限分明,5 年前发病。实验室检查无异常。由于上次皮肤科住院时的进一步建议(根据坏死性黄色瘤试验进行副蛋白血症观察),进行了免疫电泳、IgG、IgM、IgA 水平、κ轻链、λ重链检查;Bence-Jones 蛋白阴性。在诊所住院期间,给予 32mg od 的甲基强的松龙、血管药物和局部皮质类固醇治疗,取得了良好的治疗反应。
我们描述了一例具有典型临床和组织学特征的脂性渐进性坏死,无糖尿病性脂性渐进性坏死性脂膜炎,尽管长期怀疑,但未进行适当治疗,效果不佳。